Seattle Children’s Hospital researcher understands Kent teen’s need for treatment

Kent teenager Jayne Johnson knows intimately the experience of living with Severe Chronic Neutropenia Kostmann’s Syndrome – a condition that means her bone marrow doesn’t work right, and her immune system is failing.

But Dr. Akiko Shimamura of Seattle Children’s Hospital understands the disease on a level that is nearly as intimate.

She has spent years studying it.

Shimamura, a doctor and researcher who started the Bone Marrow Failure program at Children’s Hospital, is a specialist in the realm of a host of disorders involving marrow. She’s also one of Johnson’s doctors, and so has been learning about the disease – and the girl – over the past several years.

“It’s been so sad to watch her go through to this state,” Shimamura said of Johnson, who has gradually been losing ground to her illness, due to her medication not working effectively anymore, and her bone marrow continuing to fail.

While Johnson doesn’t have the cancer that usually heralds the big gun in treatment – a stem-cell transplant to give her a new immune system – Shimamura said the teenager’s quality of life is at a point where the transplant is a viable option. It was a determination made after much discussion with health-care providers, and with Johnson and her family.

“At this point, she’s in such a bad place in her health, we made the decision to go in and say, ‘it’s time,’” Shimamura said of Johnson, who now lives in constant pain, too wiped out to leave her home. “She’s debilitated now, she’s at home, she’s depressed. It’s hindered her life.”

Shimamura explained that Johnson will be getting her transplant through umbilical-cord blood, of which her doctors found a workable match. This was a different outcome than the match Johnson hoped she would get through the bone-marrow registry for a transplant. Unfortunately after years of searching, a viable marrow donor could not be found.

Out of the two options, bone marrow is the more preferable for a transplant. It’s genetically more closely matched to its host, meaning less chance of rejection, and there is a lot more of it – meaning a living host whose bones are always producing more.

But there are more genetic varieties of umbilical-cord blood available than there are marrow.

Shimamura said the umbilical-cord blood registry is the bigger of the two registries, because umbilical-cord blood is easier for people to donate.

“The nice thing about cord blood is you are taking material that otherwise would just be thrown away,” Shimamura explained, noting people are more willing to donate it, when asked.

Bone marrow, on the other hand, involves a specific medical procedure to extract it, after much testing. It’s not something you’d just give up during another medical event, as cord blood is.

There is also the fact that doctors have been doing bone-marrow transplants longer than they have been doing stem-cell transplants from cord blood. More experience means a higher rate of success.

“There are more complications with cord blood,” Shimamura said.

But there are bright spots in the picture as well.

For instance, there is the fact that Johnson is lucky enough to be living in the Puget Sound region. Seattle, which has a multitude of world-class medical facilities, also has been an epicenter for research into diseases like the one Johnson is grappling with.

And then there is another factor.

Johnson herself.

“She’s a feisty one,” Shimamura said. “If anyone can make it, I say it’s Jayne.”